What is Paget's disease of the breast?

Paget’s disease of the breast is a condition that causes an eczema-like change to the skin of the nipple. Nine out of 10 women who have Paget’s disease (90%) will also have breast cancer. The underlying breast cancer may be ductal carcinoma in situ (DCIS) or an invasive breast cancer. In DCIS, the cancer cells are completely contained within the milk ducts. Invasive breast cancer starts in the ducts or lobes of the breast, and spreads into surrounding breast tissue. It may help to read our information about breast cancer and ductal carcinoma in situ (DCIS).

Paget’s disease affects about one to two out of every 100 women with breast cancer (1–2%). It's most common in women aged 50 or over, but can occur at a younger age. It can affect men, but this is extremely rare.

Signs and symptoms of Paget's disease

The first symptom of Paget's disease is usually a scaly, red rash affecting the nipple. The dark area of skin surrounding the nipple (the areola) may also be affected. The rash doesn't go away and may become sore.

The skin of the nipple and areola may be inflamed. There may also be crusting, bleeding and ulceration. Some women have an itching or burning sensation. Fluid (discharge) may leak from the abnormal area. The nipple may turn inwards (be inverted).

There may or may not be a lump in the breast. Around half of all women who have Paget’s disease (50%) have a breast lump that can be felt at the time the disease is diagnosed.

How Paget's disease is diagnosed

Paget’s disease can be confused with other skin conditions such as eczema, dermatitis and psoriasis, because they can look very similar. This can make Paget’s disease difficult to diagnose on appearance alone.

Paget’s disease usually affects the nipple first and then the surrounding tissue. Other skin conditions usually affect the areola first, and then spread into the nipple.

Mammogram (breast x-ray)

A mammogram is a low-dose x-ray of the breast tissue. You‘ll be asked to remove the clothes from the top of your body, including your bra. The radiographer will then position you so that each breast is placed in turn on the x-ray machine and gently but firmly compressed (flattened) with a clear, plastic plate.

The breast tissue needs to be compressed to keep the breast still and to get the clearest picture with the lowest amount of radiation possible. Most women find this uncomfortable, and for some women it is painful for a short time while the breast is being compressed. You will need to stay still for less than a minute while the x-ray is taken. Usually two mammograms are taken of each breast from different angles. The appointment takes about 30 minutes.

Ultrasound scan

This test uses sound waves to produce a picture of the breast tissue. The person doing the scan puts a gel on to your breast and moves a small hand-held device around the area. The sound waves are converted into a picture using a computer. The test is painless and takes 5–10 minutes.

Biopsy

This is the main test for cancer cells below the surface of the skin. A local anaesthetic is given to numb the area. A small sample of skin and underlying breast tissue is then taken and sent to the laboratory to be examined under a microscope. The biopsy may be taken at the same time as an ultrasound to make sure that it's taken from the correct area.

Imprint or scrape cytology

Cells from the affected area can be scraped, or pressed, onto a glass slide, to be examined under a microscope.

Treatment for Paget's disease

The main treatment for Paget’s disease is surgery. Other treatment depends on whether the underlying breast cancer is DCIS or an invasive cancer. Some people don't need any further treatment after surgery. Others may have a combination of treatments, including radiotherapy, hormonal therapy, chemotherapy or a targeted therapy.

Surgery for Paget's disease

The type of operation you have will depend on how much the breast is affected, the position of the cancer and your preference. Your surgeon and breast care nurse will explain more about the best type of operation for you.

Mastectomy

Mastectomy is an operation to remove all of the breast. It will be suggested if the cancer affects a wide area, or if there's DCIS in different parts of the breast. Some women may have a new breast shape formed (breast reconstruction) at the same time as the mastectomy. But this may not be suitable for everyone, and breast reconstruction can be done after treatment is finished.

Wide local excision (breast-conserving surgery)

Wide local excision is a smaller operation. The surgeon removes the affected breast tissue, along with some surrounding normal tissue and the nipple and areola. It’s usually possible if there's only a small area of cancer and the surgeon thinks the appearance of your breast will still be good and acceptable to you after surgery.

Radiotherapy for Paget's disease

Radiotherapy treats cancer by using high energy x-rays to destroy the cancer cells, while doing as little harm as possible to the healthy cells.

You will usually have radiotherapy to the remaining breast tissue after breast-conserving surgery. It’s given to reduce the risk of the cancer coming back in the breast. If the cancer is invasive, some women who have a mastectomy may be given radiotherapy to the chest afterwards.

Radiotherapy is given as a series of short daily sessions, usually Monday to Friday, with a rest at weekends. Each treatment takes 10–15 minutes. A course of radiotherapy for breast cancer usually lasts three weeks. Your doctor or nurse will discuss the treatment and possible side effects with you. The skin in the treatment area may become red or sore or, if you are dark skinned, it may become darker. You’ll be given advice on how to look after your skin. You’re likely to feel tired during treatment and this may continue for a month or two after it. Get plenty of rest, but balance this with gentle, regular exercise, such as short walks, which will give you more energy.

Side effects usually gradually disappear once your treatment has finished.

Hormonal therapy for Paget's disease

Some breast cancer cells have oestrogen receptors on their surfaces. This is called oestrogen receptor positive (ER positive) breast cancer. Oestrogen is a hormone produced in the body. It can encourage some breast cancer cells to grow.

If you have ER positive breast cancer, you'll be prescribed hormonal therapy to reduce the risk of cancer coming back and to protect the other breast. You usually have it for up to five years. There are different types of hormonal therapy and they work in different ways. Some hormonal therapies reduce the amount of oestrogen in the body, while others prevent oestrogen from reaching the cancer cells.

Chemotherapy for Paget's disease

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It's not used to treat an underlying DCIS, but it may be given if the cancer is invasive.

You will have the chemotherapy drugs given by injection into a vein (intravenously) or as tablets. Chemotherapy into the vein is given as a session of treatment, usually over a few hours. This is followed by a rest period of a few weeks, so your body can recover from any side effects of the treatment.

Side effects of chemotherapy can often be well controlled by medicines. They usually improve when treatment is over. Side effects the drugs may cause include increased risk of infection, tiredness (fatigue), feeling sick (nausea) and hair loss.

Always let your doctor or nurse know about any side effects you have. They can usually suggest ways to control or improve them.

Targeted therapy for Paget's disease

Targeted therapies (sometimes called biological therapies) are drugs that work differently from chemotherapy. The main targeted therapy used in breast cancer is trastuzumab (usually called Herceptin®).

Some breast cancer cells have too much of a protein called HER2 (human epidermal growth factor 2) on their surface. The extra HER2 receptors stimulate the cancer cells to divide and grow. Herceptin locks on to the HER2 protein. This blocks the receptor and stops the cells from dividing and growing.

Herceptin can affect the way the heart works, so you will have tests to check the health of your heart first.

Herceptin is usually given once every three weeks for a year. You may have it with chemotherapy or after chemotherapy has finished. It’s given as a drip (infusion) into a vein in your hand or arm. Side effects can include flu-like symptoms, diarrhoea and headaches, but these tend to be mild.

Research - clinical trials for Paget's disease

Your doctor may ask you to take part in a clinical trial. Cancer doctors use clinical trials to assess new treatments.

If you decide to take part in a trial, your doctor will discuss the treatment with you so that you have a full understanding of the trial and what it means to take part. You may decide not to take part or to withdraw from a trial at any stage. You will then receive the best standard treatment available.

Your feelings

You may have many different emotions, including anger, resentment, guilt, anxiety and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their condition.

Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Our cancer support specialists can give you information about counselling in your area.

Featured group

Breast cancer is the most common cancer in women in the UK. Men can also develop breast cancer, although this is rare. This group is for anyone affected by breast cancer to get together, ask questions, share experiences and support each other.

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